Anti-plasma cell therapy in patients with monoclonal gammopathy-associated refractory pure red cell aplasia: A report of three cases and literature review

Le résumé fourni par la source

Acquired pure red cell aplasia (aPRCA) is a clinical syndrome that may be secondary to a diverse array of diseases. It is rarely secondary to monoclonal gammopathy of undetermined significance (MGUS). Currently, there is no consensus on when to initiate anti-plasma cell therapy. This study reports three cases of patients with MGUS-associated aPRCA who responded to anti-plasma cell therapy after failing conventional immunosuppressive treatment. Patient 1 achieved complete remission after treatment with the BD (bortezomib and dexamethasone) and BRD (bortezomib, lenalidomide, and dexamethasone) regimens. She subsequently received lenalidomide as maintenance treatment. She has remained in treatment-free survival (TFS) for 22 months to date. Patient 2 achieved complete remission after treatment with the BRD regimen and was maintained with lenalidomide. She has maintained TFS for 16 months so far. Patient 3 was treated with the BD regimen and became transfusion-independent, but relapsed after treatment discontinuation due to COVID-19 infection.This study suggests that anti-plasma cell therapy may be an effective strategy for aPRCA patients unresponsive to conventional immunosuppressive therapy and exhibiting monoclonal protein (M-protein). However, further studies are needed to explore the optimal timing and duration of such therapy.

Ce résumé expose les affirmations des auteurs. BNTIC ne l’interprète pas comme une validation indépendante des résultats.